Diauretics
Management and prognosis
The pathophys
The Nephrics
The Nephrics 2.0 and improved
100
A 43-year-old woman undergoes routine laboratory studies shortly after beginning a new hypertension medication. She has a history of diabetes and hypertension and had previously been prescribed lisinopril, which failed to adequately control her blood pressure. Today’s laboratory results are compared with the values prior to starting the new medication, as shown in the table.
What medication is most likely responsible for the changes in this patient's laboratory test results?
A Amlodipine
B Furosemide
C Hydrochlorothiazide
D Metoprolol
E Spironolactone
C Hydrochlorothiazide
- Thiazide diuretics are a first-line treatment for hypertension, and common adverse effects include hyperGlycemia, hyperLipidemia, hyperUricemia, and hyperCalcemia (hyperGLUC).
100
A 4-year-old girl is brought to the physician because of increasing swelling around her eyes and over both her feet for the past 4 days. During this period, she has had frothy light yellow urine. Her vital signs are within normal limits. Physical examination shows periorbital edema and 2+ pitting edema of the lower legs and ankles. A urinalysis of this patient is most likely to show which of the following findings?
A Muddy brown casts
B Epithelial casts
C Fatty casts
D RBC casts
E Waxy casts
F WBC casts
G Hyaline casts
Fatty casts
Fatty casts are very common in the urinary sediment of patients with nephrotic syndrome. Massive proteinuria (> 3.5 g/24 h) results in low serum albumin, leading to reduced capillary oncotic pressure, thereby causing edema secondary to fluid leaking into tissue. Consequently, the liver increases all synthetic activity (involving albumin as well as other macromolecules, such as lipids) to compensate. Some of these excess lipids are reabsorbed by the proximal tubular epithelial cells. Once the cytoplasm becomes engorged, chunks of the cell can slough off into the tubular lumen, leading to the classic fatty casts on urinary sediment.
Patients with nephrotic syndrome are at high risk of developing infections (due to urinary loss of IgG), hypocalcemia (due to vitamin D deficiency), and venous thrombus formation (due to urinary loss of antithrombin III and loss of fluid into the extravascular space).
Patients with nephrotic syndrome are at high risk of developing infections (due to urinary loss of IgG), hypocalcemia (due to vitamin D deficiency), and venous thrombus formation (due to urinary loss of antithrombin III and loss of fluid into the extravascular space).
100
A previously healthy 42-year-old man comes to the emergency department with hemoptysis, fatigue, and breathlessness for 3 weeks. He has bilateral inspiratory crackles and bilateral pitting edema in his knees. Urinalysis reveals dysmorphic RBCs (acanthrocytes) and moderate protein. The serum creatinine is 3.1 mg/dL. X-ray of the chest shows diffuse opacities in both lower lung fields.
Which of the following is the most likely diagnosis?
A Acute poststreptococcal glomerulonephritis
B Alport syndrome
C Goodpasture syndrome
D Granulomatosis with polyangiitis
E IgA nephropathy
F Membranoproliferative glomerulonephritis
C Goodpasture syndrome
High-Yield Summary
- Goodpasture syndrome is a rare autoimmune disorder in which antibodies directed against GBM antigen cause a rapidly progressive glomerulonephritis. Clinical presentation includes hemoptysis, urine dysmorphic RBCs, and renal failure.
Step 1: Disease Diagnosis
Combined hemoptysis, hematuria, urine dysmorphic RBCs (acanthrocytes), and renal failure (elevated serum creatinine) supports a diagnosis of Goodpasture syndrome, a rare autoimmune disorder in which antibodies directed against glomerular basement membrane (GBM) antigen cause a rapidly progressive glomerulonephritis. Hemoptysis occurs if antibodies are deposited in the alveolar basement. Diagnosis of Goodpasture syndrome can be confirmed by the presence of anti-GBM antibodies in the blood and a lung or kidney biopsy specimen that demonstrates a linear staining pattern on immunofluorescence, as shown in the image of a kidney biopsy.
100
A 36-year-old man comes to the emergency department because of worsening leg swelling for 2 days. Physical examination reveals bilateral pitting lower limb edema. Laboratory tests reveal elevated serum blood urea nitrogen and creatinine. Urinalysis shows RBC and RBC casts. A kidney biopsy specimen shows many glomeruli similar to the one shown.
Which of the following is the most likely diagnosis?
A Focal segmental glomerulosclerosis
B Hemolytic-uremic syndrome
C Minimal change disease
D Post infectious glomerulonephritis
E Rapidly progressive glomerulonephritis
E Rapidly progressive glomerulonephritis
High-Yield Summary
- RPGN causes epithelial crescents in glomeruli, hematuria, RBC casts, and progressive renal failure. It can be primary or secondary to disorders like lupus nephritis.
Step 1: Disease Diagnosis
This patient has worsening kidney function, hematuria, RBC casts, and epithelial crescents seen on renal biopsy (see figure arrows), all suggesting rapidly progressive glomerulonephritis (RPGN). This should be suspected in any patient with both hematuria, proteinuria, and rapidly declining renal function. Most types of RPGN can lead to rapid loss of renal function and respond to immunosuppressive therapy, so RPGN is one of the uncommon reasons to do an urgent renal biopsy.
RPGN can be primary (without other renal disease) or due to other glomerular diseases like lupus nephritis, IgA vasculitis, or cryoglobulinemia. Less commonly, crescents can be seen with primary glomerular disease like membranoproliferative glomerulonephritis, membranous glomerulonephritis, or IgA nephropathy.
100
A 50-year-old woman comes to the office because of a sudden increase in leg edema. She has long-standing type 2 diabetes mellitus treated with metformin. Her current blood pressure is 132/92 mm Hg. Physical examination shows 3+ edema to mid-calf bilaterally. Laboratory results show a glycosylated hemoglobin level of 8% and a serum creatinine concentration of 2.3 mg/dL. Urinalysis shows 4+ protein with no blood, cells, or casts. Urine studies estimate 5.5 g of protein per 24 hours. Renal biopsy specimen shows nodular sclerosis.
Which of the following histologic findings would have most likely been seen at an earlier stage of this patient’s renal disease?
A Basement membrane thickening
B Intramembranous immune deposits
C Mesangial IgA deposits
D Subendothelial immune deposits
E Subepithelial immune deposits
A Basement membrane thickening
Basement membrane thickening
High-Yield Summary
- Diabetic nephropathy causes nephrotic range proteinuria, and biopsy commonly shows nodular sclerosis.
- Early diabetic nephropathy shows microalbuminuria, and pathology shows a thickened GBM.
Early diabetic nephropathy is characterized by thickening of the glomerular basement membrane (GBM) and by diffuse mesangial cellular hyperplasia. The GBM thickening is diffuse and not due to surrounding immune deposits because diabetic nephropathy is not an immune-mediated GN. Early diabetic nephropathy also shows small amounts of proteinuria (microalbuminuria) detectable on urine albumin testing. If caught early, diabetic nephropathy at this stage (30 to 300 mg albumin/24 hr) is reversible with angiotensin-converting enzyme inhibitors.
200
A 54-year-old man with congestive heart failure comes to his physician with crackles in his lower lung fields and bilateral pedal edema. His potassium level is 6.2 mEq/L.
Which of the following medications would best correct the patient’s electrolyte abnormality and fluid overload?
A Amiloride
B Captopril
C Furosemide
D Spironolactone
E Triamterene
C Furosemide
- Furosemide is a potassium-wasting loop diuretic that decreases fluid overload in patients with congestive heart failure.
Loopdiuretics
Furosemide, bumetanide, torsemide
MECHANISM
Sulfonamide loopd iuretics. Inhibit cotransport system(Na+/K+/2Cl−)of thick ascending limb of loop of Henle. Abolish hypertonicity of medulla, preventing concentration of urine. Associated with PGE (vasodilatory effect on afferent arteriole); inhibited by NSAIDs. Ca2+excretion.
ADVERSEEFFECTS
Ototoxicity, Hypokalemia, Hypomagnesemia, Dehydration, Allergy(sulfa), metabolic Alkalosis, Nephritis(interstitial), Gout.
200
A 27-year-old man comes to the physician because of cough and runny nose for 2 days, and cola-colored urine for 1 day. The family history is negative for renal disease. The physical examination is normal. Urinalysis reveals 2+ proteinuria and 4+ hematuria with 1 to 2 RBC casts.
Which of the following disorders has urine findings most similar to those in this patient?
A Amyloidosis
B Analgesic nephropathy
C Contrast nephropathy
D Focal segmental glomerulosclerosis
E IgA vasculitis
F Membranous nephropathy
E IgA nephropathy
High-Yield Summary
- Patients with nephritic syndrome present with hematuria, RBC casts in the urine, and mild proteinuria.
- Both IgA vasculitis and IgA nephropathy present with nephritic syndrome and are considered to be related diseases that share similar renal pathology.
200
A 26-year-old woman comes to the clinic because of facial rash, arthralgias, and swollen feet. Temperature is 37.0°C (98.6°F), and blood pressure is 152/88 mm Hg. Physical examination shows an erythematous rash on the forehead and cheeks. There is 3+ pedal edema and joint swelling in the metacarpophalangeal joints bilaterally. Serum creatinine is 2.6 mg/dL. Urinalysis shows blood and protein with dysmorphic RBC seen on microscopy. Renal biopsy results are shown.
Courtesy of A. Subhawong.
The pathology seen in this image is most likely caused by which of the following?
A Alport syndrome
B Focal segmental glomerulosclerosis
C Lupus nephritis
D Minimal change disease
E Postinfectious glomerulonephritis
C Lupus nephritis
High-Yield Summary
Patients with systemic lupus erythematosus are likely to develop nephritis because circulating immune complexes in the serum deposit in the glomerulus and activate complement, leading to leukocyte infiltration and membrane damage. The damage is observed on biopsy as cellular proliferation and thickening of the glomerular capillary wall.
Does not say, but I think this is "diffuse proliferative glomerulonephritis" It does say that there are wire looping's in the left lower portion.
200
A 6-year-old boy is brought to the office by his parents, who have observed swelling in his face and legs over the past day. His blood pressure is 100/70 mm Hg. Physical examination shows edema in the periorbital area and bilateral lower extremities. Urinalysis shows heavy protein and no red blood cells.
If a renal biopsy specimen were obtained, which of the following would be the most likely on immunofluorescence of this patient’s renal specimen?
A Absence of immunoglobulin deposits
B IgG and C3 along the capillary wall and in the mesangium
C Linear IgG deposition along the glomerular basement membrane
D Mesangial and subendothelial IgM, C3 λ, and κ light chains
E Wire loop lesions with subendothelial immune complex deposits
A Absence of immunoglobulin deposits
High-Yield Summary
- MCD is the most common cause of childhood nephrotic syndrome.
- The renal pathology appears normal on light microscopy and immunofluorescence, but effacement of podocyte foot processes is visible on electron microscopy.
200
An 11-year-old girl comes to the physician with her mother because of a 2-day history of passing “cola-colored“ urine. During the past week, her mother noticed episodes of facial swelling. The patient had a rash on her face about 4 weeks ago. A renal biopsy after immunofluorescence is shown. Which of the following is the most likely diagnosis?
A Poststreptococcal glomerulonephritis
A Poststreptococcal glomerulonephritis
Poststreptococcal glomerulonephritis (PSGN) is an acute glomerular inflammation caused by the deposition of immune complexes along the glomerular basement membrane and mesangium in response to infection with nephritogenic strains of group A streptococci (GAS). PSGN classically occurs in children 1–4 weeks after infection (e.g., impetigo) and manifests with periorbital (or generalized) edema and gross hematuria (“cola-colored” urine), both of which are seen in this patient. In addition, PSGN can also manifest with hypertension, azotemia, and proteinuria. Granular deposits of IgG, IgM, and C3 create a characteristic “lumpy-bumpy” appearance on immunofluorescence of a renal biopsy specimen.
300
A 70-year-old man with a history of poorly controlled congestive heart failure comes to the physician for a follow-up examination. At his previous visit 4 months ago, a new drug was added to his treatment regimen. He reports that his dyspnea and peripheral edema have improved. His pulse is 70/min and blood pressure is 110/80 mm Hg. Physical examination shows bilateral, mildly tender enlargement of breast tissue. This patient's physical examination finding is most likely caused by a drug that acts at which of the following sites in the kidney?
A Proximal convoluted tubule
B Juxtaglomerular apparatus
C Efferent arteriole
D Early distal convoluted tubule
E Thick ascending limb
F Cortical collecting duct
F. Cortical collecting duct
Spironolactone, which is a competitive aldosterone receptors antagonist, acts on the principal cells in the cortical collecting ducts. It is used to reduce morbidity and mortality in patients with heart failure. Spironolactone inhibits the binding of androgens to their receptors as well as the activity of enzymes involved in androgen synthesis (e.g., 17,20-desmolase, 17α-hydroxylase). It also increases the peripheral conversion of testosterone to estradiol and displaces estradiol from SHBG. This combination of antiandrogenic and pro-estrogenic effects can cause gynecomastia and impotence in men.
Eplerenone is an aldosterone receptor antagonist with a greater specificity for the mineralocorticoid receptor than spironolactone; it has a lower incidence of endocrine side effects.
300
A 20-year-old man comes to the physician because of dark urine and decreased urine output for 2 days. He had a skin infection that required antibiotic treatment 3 weeks ago but stopped the antibiotics early because the infection had resolved. His blood pressure is 140/90 mm Hg. Physical examination shows periorbital edema bilaterally. A photomicrograph of a renal biopsy specimen is shown. Further evaluation of this patient is most likely to show which of the following findings?
A Mutation in type IV collagen
F Elevated Anti-DNase B titer
Elevated anti-DNase B titers indicate a recent infection by GAS. Infection with nephritogenic strains of GAS results in subepithelial immune complex deposition within the glomerular basement membrane (type III hypersensitivity reaction), which activates the complement system. This results in glomerular inflammation and nephritic syndrome, which is then known as poststreptococcal glomerulonephritis (PSGN). Although PSGN is more common in young children and older adults, it can occur at any age. Adults are more likely to suffer from lasting kidney damage after PSGN, while the vast majority (> 90%) of children will fully recover.
Laboratory studies in this patient would also show a decreased C3 complement level due to increased complement consumption. ASO titers may also be elevated but may be falsely negative, especially after a GAS skin infection, as in this case.
300
A 62-year-old woman with type 2 diabetes mellitus comes to the physician because of a 3-month history of fatigue and weakness. Her hemoglobin A1c concentration was 13.5% 12 weeks ago. Her blood pressure is 152/92 mm Hg. Examination shows lower extremity edema. Serum studies show:
K 5.1
Phosphorus 5.0
Ca 7.8
Urea nitrogen 60
Creatinine 2.2
Which of the following is the most appropriate parameter for early detection of this patient’s renal condition?
A Urinary red blood cell casts
B Serum total protein
C Urinary albumin
D Serum urea nitrogen
E Serum creatinine
C Urinary albumin
Microalbuminuria is the earliest diagnostic sign of diabetic nephropathy. Progression of glomerular damage (nodular glomerulosclerosis) leads to increased urinary loss of albumin, which can manifest as nephrotic syndrome. The extent of albuminuria in patients with diabetic nephropathy also correlates with the risk of future cardiovascular events. ACE inhibitors can prevent the progression of albuminuria in patients with diabetic nephropathy.
300
A 47-year-old woman comes to the physician because of a 2-week history of gradually worsening facial and lower extremity swelling. She has had a 4-kg (8.8-lb) weight gain during this time. Her blood pressure is 150/88 mm Hg. Examination shows periorbital edema and 2+ pretibial edema bilaterally. A 24-hour collection of urine shows 4.0 g of proteinuria. Microscopic examination of a kidney biopsy specimen shows thickening of the glomerular basement membrane. Electron microscopy shows dense subepithelial deposits. Further evaluation is most likely to show which of the following?
A Anti-phospholipase A2 receptor antibodies
A Anti-phospholipase A2 receptor antibodies
Anti-phospholipase A2 receptor antibodies are found in primary membranous nephropathy. Deposition of antibodies between podocytes and the glomerular basal membrane (GBM) causes thickening of glomerular capillary loops and the GBM, which in turn leads to nephrotic syndrome. On electron microscopy, the subepithelial immune deposits are reflected in a “spike and dome” appearance.
Secondary membranous nephropathy can be caused by infection (e.g., HBV, HCV) or systemic lupus erythematosus.
300
A 17-year-old girl comes to the clinic because of swollen legs for 2 days. In the past 2 days, her urine has appeared brown and frothy. Three weeks ago, she had a sore throat and mild fever that resolved spontaneously. Blood pressure is 138/88 mm Hg. Physical examination shows 3+ edema to the midcalf. Urinalysis shows 3+ blood.
Which of the following is the most likely cause of this patient’s condition?
A Fused glomerular visceral epithelial cells with normal-appearing glomeruli on light microscopy
B Mesangial IgA deposition
C Thickening of the glomerular basement membrane without immune deposits
D Thinning of the glomerular basement membrane
E Type II hypersensitivity reaction
F Type III hypersensitivity reaction
F Type III hypersensitivity reaction
High-Yield Summary
- Postinfectious GN may cause edema, proteinuria, and hematuria 2 to 3 weeks after bacterial infection such as strep throat.
- Postinfectious GN results from a type III hypersensitivity reaction with antigen-antibody complex deposition in the glomerulus.
400
A 64-year-old woman is brought to the emergency department because of a 1-week history of progressive shortness of breath, lower extremity edema, and a 4-kg (9-lb) weight gain. She has ischemic cardiomyopathy and rheumatoid arthritis. Her respirations are 27/min. Examination shows pitting edema of the lower extremities and crackles over both lower lung fields. Therapy is initiated with intravenous furosemide. After 2 hours, urine output is minimal. Concomitant treatment with which of the following drugs is most likely to have contributed to treatment failure?
A Sulfasalazine
B Ibuprofen
Ibuprofen
Nonsteroidal anti-inflammatory drugs such as Ibuprofen are commonly used in the treatment of rheumatoid arthritis. NSAIDs adversely affect renal function by inhibiting renal synthesis of prostaglandins, which help maintain renal perfusion in dehydrated states. Decreased prostaglandin synthesis (e.g., of prostaglandin E1) counteracts the therapeutic effect of furosemide and can lead to hypoperfusion of the kidneys and reduced GFR.
This patient's symptoms at the time of presentation are likely due to acute exacerbation of ischemic cardiomyopathy.
This patient's symptoms at the time of presentation are likely due to acute exacerbation of ischemic cardiomyopathy.
400
A 5-year-old boy is brought to the emergency department by his parents because of a 3-day history of fatigue, pallor, and facial puffiness. Two weeks ago, he had a sore throat and was given a course of antibiotics. His blood pressure is 138/90 mm Hg. Physical examination reveals periorbital swelling. A urine sample shows proteinuria, hematuria, and RBC casts.
Which of the following is most likely to be seen on electron microscopy (EM) of this patient’s glomeruli?
A Diffuse thickening of the glomerular basement membrane
B Hump-like deposits along the glomerular basement membrane
C Isolated effacement of the foot processes of the visceral epithelial cells
D Mesangial expansion and antigen-antibody deposits
E Thinning and splitting of the glomerular basement membrane without inflammatory cells
B Hump-like deposits along the glomerular basement membrane
High-Yield Summary
- Infection-related glomerulonephritis can present with proteinuria, hematuria, and hypertension 1 to 3 weeks after streptococcal pharyngitis.
- Histologically, infection-related glomerulonephritis is characterized by enlarged hypercellular glomeruli, with dense subepithelial humps on the basement membrane.
400
A 45-year-old woman comes to her physician because of fatigue and increasing leg swelling for the past 5 days. There is no family history of renal disease. Urine studies reveal no cells or casts but show proteinuria of 6 g/24 h.
Disruption or loss of which of the following within the kidney is most likely contributing to this patient’s proteinuria?
A Brush border
B Glomerular type IV collagen
C Heparan sulfate
D Integrins
E Parietal epithelial cells
C Heparan sulfate
High-Yield Summary
- Nephrotic syndrome occurs in some glomerular diseases and shows heavy proteinuria of >3.5 g/24 h.
- Cases of nephrotic syndrome commonly show abnormal GBM structure, particularly loss of negatively charged heparan sulfate proteoglycans. This change increases passage of negatively charged proteins through the GBM.
400
A 56-year-old woman comes to the clinic because of swollen legs for several days. Urinalysis shows 4+ proteinuria; serum albumin concentration is 2.9 mg/dL. Renal biopsy specimen shows the findings pictured in the image in about 40%-50% of the glomeruli.
Image courtesy of Wikimedia Commons
Which of the following risk factors is most closely associated with this condition?
A Hepatitis C
B Injectable drug use
C Lymphoma
D Nonsteroidal anti-inflammatory drug use
E Staphylococcal infection
F Streptococcal infection
B Injectable drug use
High-Yield Summary
- Focal segmental glomerulosclerosis presents with clinical and urinalysis findings of nephrotic syndrome.
- Risk factors for focal segmental glomerulosclerosis include HIV, injectable drug use, heroin use, morbid obesity, and, less commonly, lupus.
400
A previously well 30-year-old man comes to his physician’s office because of a persistent cough for several months. He has noticed blood mixed in the mucus and has also had episodes of dark red urine. Urinalysis is positive for 16 RBCs/hpf with several RBC casts. The serum antinuclear antibodies and antineutrophilic cytoplasmic antibody are negative.
Which of the following is the most likely cause of this patient’s condition?
A Effacement of foot processes with normal immunofluorescence
B Glomerular mesangial immune complex deposits
C Glomerular subendothelial immune complex deposits
D Linear antibody deposition along the glomerular basement membrane
E Mutation of type IV collagen
D Linear antibody deposition along the glomerular basement membrane
- Goodpasture syndrome leads to hemoptysis, renal failure, and hematuria.
- In Goodpasture syndrome, biopsy specimens show a smooth, linear pattern on immunofluorescence and crescent formation under light microscopy.
500
A new drug is developed that inhibits the sodium-potassium-chloride cotransporter in the ascending loop of Henle. Which of the following sets of changes is most likely to occur after 2 days of therapy with this drug?
Serum pH Jugular venous pressureA No change Increase
B No change No change
C Decreased Decreased
D Increased Decreased
E Decreased Increased
D
Increased serum pH and decreased JVP occur when the Na-K-Cl cotransporter in the ascending loop of Henle is inhibited. Inhibiting this cotransporter impairs Na+ reabsorption, which causes relative hypovolemia. The typical response is contraction alkalosis (increased serum pH, reduced JVP). The inability to reabsorb K+ in the ascending loop also results in a higher luminal concentration in the collecting tubule, where K+ ions are absorbed at the expense of H+, further contributing to metabolic alkalosis.
500
Getting this question wrong DOES NOT count towards your negative points if you get it wrong but it counts towards your positive if gotten right.
HOWEVER you can wager 500 points here if you want, if you get it wrong you will loose 500 points but if you get it right you will gain 1000!!!!
Name how to manage/treat all of this layers of nephrotic syndrome.
Edema(3 ways)
Proteinuria(1 way)
Dyslipidemia(1 way)
Hypercoagulability( 1 way)
Risk of infection( 1 way)
Edema
1 Dietary sodium restriction
2 Fluid restriction
3 Diuretic therapy
Proteinuria
1 RAAS/ARB
2 Eat protein
Dyslipidemia
1 Statin (or others)
Hypercoagulability
1 Heparin/warfarin/?DAOCs?(lacking evidence)
Hypocomplementemia
1 Vaccination is what ambos says.
2 Willing to take prophylactic antibiotics, but amboss does not say that and I will not put that on my test.
500
A 48-year-old man comes to the emergency department because of sudden right flank pain that began 3 hours ago. He also noticed blood in his urine. Over the past two weeks, he has developed progressive lower extremity swelling and a 4-kg (9-lb) weight gain. Examination shows bilateral 2+ pitting edema of the lower extremities. Urinalysis with dipstick shows 4+ protein, positive glucose, and multiple red cell and fatty casts. Abdominal CT shows a large right kidney with abundant collateral vessels and a filling defect in the right renal vein. Which of the following is the most likely underlying cause of this patient's symptoms?
A Factor V Leiden
C Loss of antithrombin III
Nephrotic syndrome is characterized by a massive renal loss of protein due to structural damage of the glomerular filtration barrier. This nonselective proteinuria includes the loss of antithrombin III, leading to a hypercoagulable state due to decreased inhibition of Factor IIa and Factor Xa. The additional loss of albumin reduces the oncotic pressure inside blood vessels, leading to fluid shifts into the extravascular space, as evidenced by this patient's signs of volume overload (weight gain, lower extremity edema). Reduced intravascular volume significantly increases the risk of hypercoagulability and subsequent thromboembolic events, such as this patient's renal vein thrombosis.
500
A 17-year-old boy is brought to the office because of decreasing hearing over the past year. Family history is significant for chronic kidney disease in his mother. Hearing evaluation confirms bilateral high-frequency sensorineural hearing loss. Laboratory tests show a serum creatinine level of 0.8 mg/dL. Urinalysis reveals 50 to 100 RBCs/hpf and 1+ proteinuria.
Which of the following is the most likely diagnosis?
A Alport syndrome
B Autosomal-dominant polycystic kidney disease
C Ehlers-Danlos syndrome
D IgA vasculitis
E Osteogenesis imperfecta
Cant see? Cant pee? Cant hear a bee?
A Alport syndrome
High-Yield Summary
- Alport syndrome (hereditary nephritis) is a genetic disorder of type IV collagen structure that may cause microscopic hematuria, progressive chronic kidney disease, hearing loss, and lens abnormalities.
500
A 27-year-old woman comes to the physician because of 3 months of increasing shortness of breath, with chest pain when she breathes in. She reports hand stiffness with pain and has noticed a rash on her cheeks when she is outside in the sun. Physical examination reveals swollen, tender metacarpophalangeal joints in both hands, decreased breath sounds at both lung bases, and a pericardial rub. Laboratory studies show:
WBC count: 8900/mm³
Hematocrit: 28.1%
Hemoglobin: 9.3 g/dL
Platelet count: 87,000/mm³
Blood urea nitrogen: 36 mg/dL
Creatinine: 2.2 mg/dL
Urinalysis shows 3+ heme and 2+ protein; 8 to 10 dysmorphic RBC/hpf are seen. X-ray of the chest shows large bilateral pleural effusions.
Which of the following renal pathologies is most likely seen on this patient’s renal biopsy?
A Linear deposits of IgG on the basement membrane
B Mesangial IgA deposits
C Necrosing tubular cells
D Pink hyaline nodules in regions of glomerular capillary loops
E Segmental sclerosis and hyalinosis
F Subendothelial glomerular immune complexes
F Subendothelial glomerular immune complexes
High-Yield Summary
- Systemic lupus may present with skin, joint, serosal, hematologic, and renal disease.
- Lupus nephritis presents with hematuria, proteinuria, and decreased renal function and shows subendothelial immune deposits.
Step 1: Disease Diagnosis
This 27-year-old woman has a multisystem disease showing photosensitive malar rash, arthritis, pleuritis (pleuritic chest pain, pleural effusions on imaging), pericarditis (friction rub), renal disease (hematuria, proteinuria, increased creatinine), anemia, and thrombocytopenia. These findings are consistent with systemic lupus erythematosus (SLE)